Feeding practices and nutrition recommendations for infants with cystic fibrosis.

Cystic fibrosis (CF) is the most common fatal genetic disorder in Caucasians and affects one in every 2,000 births (1). It is characterized by dysfunction of the exocrine glands and clinical manifestations of pulmonary disease, pancreatic insufficiency, and elevated levels of sodium and chloride in sweat. The cellular defect in CF is a transmembrane protein that regulates ion transport (2).

Proper nutrition is essential for persons with CF. Good nutritional status can influence long-term survival and quality of life (3,4) and may improve a patient's ability to fight infections, particularly those affecting the respiratory system. Aggressive medical management and early intervention have increased the median life expectancy of persons with CF from less than 2 years in 1940 to 27 to 28 years in 1989 (5).

With the cloning of the gene responsible for CF in 1989 (6,7), the scientific community has shifted the focus of its efforts to development of a safe and effective cure for CF. Gene therapy, which involves transfer of the normal gene into the lungs of a CF patient, may be a realistic option (8), but more extensive research is needed to determine the efficacy and safety of this treatment. Until there is a cure for CF, optimal nutritional status must be maintained to support the health and growth of all, especially infants.

It is still not known whether the birth weights of infants with CF are similar to those of their healthy peers. Some researchers (9-11) have reported that birth weights of infants with CF are lower than average, whereas others have found no significant differences (12,13). The poor growth exhibited by infants with CF has been attributed to a combination of factors, such as malabsorption caused by pancreatic insufficiency and increased nutritional needs secondary to lung disease (1). Today, advances in treatment for malabsorption include enzyme replacement and early nutrition intervention to normalize patterns of growth and weight gain (11,14).

Although nutrition plays an essential role in treatment of CF, feeding guidelines were not established until 1991 with publication of the Cystic Fibrosis Foundation consensus report (15). The purpose of our study was twofold: to identify the current feeding practices and nutrition recommendations made by dietitians for infants with CF between the ages of birth and 1 year and to compare survey findings with the recommendations of the consensus report.

METHODS

A 31-item questionnaire was developed to identify the nutrition recommendations of cystic fibrosis center dietitians for infants with CF. Questions were pretested for clarity by dietitians in Gainesville, Fla, who work with these infants. A panel of experts in nutrition, education, and pediatrics reviewed the questionnaire to ensure content validity.

The survey consisted of four sections. The first three used closed-ended questions to identify recommendations for infant formulas and pancreatic enzyme supplements and criteria for establishing energy intake goals. The fourth section used a five-point Likert scale to determine dietitians' attitudes about current feeding practices.

Survey packets (n = 130) were sent to dietitians in all of the cystic fibrosis centers accredited by the National Cystic Fibrosis Foundation in the United States and Canada. A cover letter explained the purpose of the research; a self-addressed stamped envelope was included in the packet to facilitate return of the completed survey. After 1 month, a second mailing was sent to …